Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature.

Abstract:

:We present the management challenge provided by a patient with kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon. A female child presented at 14 months of age with an ecchymotic swelling of her right upper arm and axilla. Subsequently, she developed profound thrombocytopenia and hypofibrinogenemia (Kasabach-Merritt phenomenon). Biopsy of the lesion revealed kaposiform hemangioendothelioma, which has been reported as the predominant pathologic diagnosis associated with Kasabach-Merritt phenomenon. To achieve involution of the lesion and preserve function of the arm, the following interventions were involved: embolization, systemic interferon, cyclophosphamide, epsilon aminocaproic acid, and compression therapy. The clinical management of this patient was formidable until we arrived at the proper combination of therapies. Multimodal intervention may be required to manage fastidious hemangioendotheliomas of childhood, achieve clinical improvement, and prevent further morbidity.

journal_name

Pediatr Hematol Oncol

authors

Blei F,Karp N,Rofsky N,Rosen R,Greco MA

doi

10.3109/08880019809014013

subject

Has Abstract

pub_date

1998-07-01 00:00:00

pages

295-305

issue

4

eissn

0888-0018

issn

1521-0669

journal_volume

15

pub_type

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